CLEFT LIP AND PALATE SURGERY: RECOMMENDED CRITERIA FOR THIRD-PARTY PAYER COVERAGE

The American Society of Plastic Surgeons (ASPS) is the largest organization of plastic surgeons in the world. Requirements for the membership include certification by the American Board of Plastic Surgery as recognized by the American Board of Medical Specialties.

ASPS represents 97 percent of the board-certified plastic surgeons practicing in the United States and Canada. It serves as the primary educational resource for plastic surgeons and as their voice on socioeconomic issues. ASPS is recognized by the American Medical Association (AMA), the American College of Surgeons (ACS) and other organizations of specialty societies.

Definitions

In the U.S.A. approximately 7 percent of children are born with craniofacial deformities. The most common of these is cleft lip and/or cleft palate. The overall incidence of cleft lip and palate is one in 700 births making this deformity the fourth most common birth defect.

Cleft Lip
A cleft lip is a birth defect that results in a unilateral or bilateral opening in the upper lip between the mouth and the nose. It causes a deformity of the lip, nose and upper jaw. ICD-9 Codes that apply: 749.10, 749.11, 749.12, 749.13, 749.14, 749.20, 749.21, 749.22, 749.23, 749.24.

Cleft Palate
A cleft palate is a birth defect characterized by an opening in the roof of the mouth, caused by a lack of tissue development. In this case, the mouth and nasal cavity, normally separated by the palate, are open to each other. The cleft can extend from the hard palate in the front of the mouth to the soft palate near the throat. Left unrepaired, a cleft palate will create feeding difficulties and lead to speech impediment, hearing loss and abnormal dental development. ICD-9 Codes that apply: 749.0, 749.01, 749.02, 749.03, 749.04, 749.20, 749.21, 749.22, 749.23, 749.24.

For a child with cleft lip or cleft lip and palate, the anomalies can be either mild or severe and can cause complex distortion of facial structures. In addition to the initial closure of the lip and palate, many patients require secondary surgery involving the lip, palate, nose and jaw. The number of operations necessary to achieve a satisfactory final result depends on the type and degree of the patient's cleft and associated problems. Adult patients who underwent repair of a cleft lip or palate before current techniques were developed may have marked residual deformities and impairments that require surgical reconstruction to approximate a normal appearance and function.

Cosmetic and Reconstructive Surgery
For reference, the following definitions of cosmetic and reconstructive surgery was adopted by the American Medical Association, June 1989.

Cosmetic surgery is performed to reshape normal structures of the body in order to improve the patient's appearance and self-esteem.

Reconstructive surgery is performed on abnormal structures of the body, caused by congenital defects, developmental abnormalities, trauma, infection, tumors or disease. It is generally performed to improve function but may also be done to approximate a normal appearance.

Primary surgery, cleft lip, ICD-9 codes: 749.1, 749.2

Cheiloplasty
Cheiloplasty, or cleft lip repair, is performed to close the opening in the lip caused by this birth defect. If the cleft is bilateral, closure may be performed on both sides simultaneously, or the surgeon may repair the lip one side at a time in separate surgeries. In some cases, when the deformity is severe, a preliminary operation to bring the two sides of the gap closer may be needed. The preliminary procedure can be either a lip adhesion (sewing the edges together without aligning the lip) or the insertion of an appliance to mechanically approximate the lip and gums. CPT codes: 13151, 40700-52, 40701-52, 42281.

The formal cleft lip repair is generally performed in a hospital under general anesthesia. There are two general surgical approaches. In a technique known as rotation-advancement lip repair, the surgeon makes an incision on either side of the nostrils, extending from the lip into the nostrils. Working through the incision, the surgeon opens the lip completely, rotates the pink outer portion downward, and advances tissue from the cheek into the defect to eliminate the cleft. In another method, referred to as triangular flap repair, the surgeon makes incisions to form small skin flaps between the lip and nose. These flaps overlap and interlock to close the defect, restore muscle function, create needed height in the flap, and form a cupid's bow. CPT: 40700, 40701, 40702.

Primary surgery, cleft palate, ICD-9: 749.0, 749.2

Palatoplasty
Palatoplasty, or cleft palate repair, is performed to close an opening in the palate. Surgeons may close the palate in one surgery when the child is about one year of age. Or, the palate may be closed in two stages. The soft palate first, followed by the hard palate.

Palatoplasty is usually performed in a hospital under general anesthesia as an inpatient procedure. Methods for repairing a cleft palate may vary widely in terms of when they are performed and what techniques are used. In a typical repair, incisions are made in the palate to provide sufficient tissue to close the defect. This tissue is moved to the mid-line or the center of the mouth to reconstruct the palate, join the muscles and provide adequate length to the soft palate. CPT codes: 15574, 42200, 42205, 42210, 42225, 42226, 42227, 44235.

Secondary surgery, ICD-9 codes: 749.0, 749.1, 749.2

Since the face grows until a child has reached maturity (girls 16 and boys 19), children born with cleft lips and palates require monitoring, and additional procedures may be required to correct residual deformities or deformities which worsen with age. CPT codes: 12051, 13150, 13151, 14060, 40720, 40761, 42215, 42220.

Patients with cleft lip deformities also have distortion of the nose. Cleft lip rhinoplasty is necessary to improve nasal function and correct the distortion. In the case of a severe nasal deformity, reconstructive rhinoplasty may be done in the child's early years. However, in other cases it is recommended that the operation be performed in the child's middle teenage years, when the nose has attained its maximum growth. Secondary surgery to achieve optimum reconstruction is common. CPT codes: 30130, 30140, 30460, 30462, 30520.

Repair of a complete cleft palate, one that extends from the lip to the throat, is generally performed in two operations. However, later revisions are often needed by children because of scarring and impaired growth of the palate. Communication (fistula) between the oral cavity and the nose or maxillary sinus is a sequela of cleft palate procedures and requires surgical closure. An additional operation, a bone graft commonly from the skull, hip or rib, may be required to replace missing bone in the roof of the mouth or gums (alveoloplasty), CPT codes: 21210, 21230, 21235, 30580, 30600, 42210, 42215, 42220, 42225, 42226, 42227, 42235, 42260, 42281.

Cleft palate patients may also have abnormal movement of the speech mechanism in the back of the throat. As they grow older and begin to speak, air may escape from the nostrils in an abnormal way and cause hypernasality. A surgical procedure known as a pharyngeal flap-palatoplasty is done to correct this deformity and permit normal speech. CPT codes: 42145, 42225, 42226.

Anomalies of the upper jaw (maxilla) develop as well, sometimes requiring surgical correction in the teenage years. If the maxilla is deficient (hypoplastic), it may require expansion or realignment by osteotomy to correct the malocclusion ( abnormal jaw relation). CPT: 21141, 21142, 21143, 21145, 21146, 21147, 21206.

When cleft lip and palate and secondary deformities are repaired, the indications should be documented by the surgeon in the history and physical and reiterated in the operative note. Chart documentation of the presence of a cleft lip or palate or other secondary deformity should qualify a procedure as medically necessary and, therefore, eligible for coverage.

Photographs are usually taken to document the pre-operative condition and aid the surgeon in planning surgery. In some cases they may record physical signs; however, they do not substantiate symptoms and should only be used by third-party payers in conjunction with less subjective documentation. In circumstances when photographs may be useful to a third-party payer, the plastic surgeon should provide them. The patient, however, must sign a specific release, and confidentiality must be honored. It is the opinion of ASPS that a board-certified plastic surgeon should evaluate all submitted photographs.

Initial repair of cleft lip and palate deformities is generally performed at an early age, but secondary surgery may be required as the patient grows older and the lip, palate, nasal and jaw structures grow and develop. It is the position of the American Society of Plastic Surgeons that both initial and secondary procedures for treatment of cleft lip and palate birth defects should be compensable by third party payers, regardless of the patient's age.

Clasper, R. "A combined obturator and expansion appliance for use in patients with patent oral-nasal fistula." Bristish Journal of Orthodontics, 22(4): 357, Nov. 1995.

Cronin, T.D. et al. "Bilateral Clefts," In: Plastic Surgery, McCarthy, J.C. Vol. 4, p. 2653, W.B. Saunders: 1990.

Furlow, L.T. "Flaps for Cleft Lip and Palate Surgery." Clinics in Plastic Surgery, Vol. 17, p. 633, 1990.

Jackson, I.T., et al. "Secondary Deformities of Cleft Lip, Nose and Palate." In: Plastic Surgery, McCarthy, J.G. Vol. 4 p. 2771, W.B. Saunders, 1990.

McComb, H. " Primary Correction of Unilateral Cleft Lip & Nasal Deformity: A 10 year review." Plastic and Reconstructive Surgery, 75: 791, 1985.

Millard, D. R. "Unilateral Cleft Lip Deformity" In: Plastic Surgery, McCarthy, J. G. Vol 4 p. 2627, 1990.

Randell, P. "A triangular flap operation for the primary repair of unilateral clefts of the lip. Plast. Reconstr. Surg., 23:331, 1959.

Smith, W.P. " Primary Closure of the Cleft Alveolus: A Functional Approach." British Journal of Oral & Maxillofacial Surgery, 33: 156-165, 1995.

Thaller, S. "Microform Cleft Lip Associated with a Complete Cleft Palate." Cleft Palate-Craniofacial Journal, Vol. 32, No. 3, pp. 247-250, May, 1995.